Eye Involvement and Management in Inherited Epidermolysis Bullosa

Bachir, Yasmine; Daruich, Alejandra; Marie, Couanon; Robert, Matthieu P.; Bremond-Gignac, Dominique

doi:10.1007/s40265-022-01770-8

Cited by 2 publications

(1 citation statement)

References 64 publications

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“…При БЭ могут быть поражены как кожа век, так и слизистая глаз. Повреждение роговицы, связанные с буллезным эпидермолизом обычно очень болезненны [15]. Пациенты с некоторыми типами БЭ испытывают сильный зуд, который приводит к появлению расчесов и повреждению кожи, что увеличивает воспаление и стимулирует нервные волокна, что в свою очередь способствует образованию обширных раневых поверхностей и усилению зуда [16].…”

Section: Introductionunclassified

The problem of pain and its assessment in children with epidermolysis bullosa

Troitskaya,

Golyaeva,

Orlova

2024

APPS

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Goal. Currently, there is an increasing interest in the treatment and improvement of the quality of life of patients with rare chronic diseases, and one of the urgent issues remains the problem of pain syndrome assessment and prevention. The purpose of the review article was to study the problem of pain and its assessment in children with epidermolysis bullosa. Theoretical justification. In the article, the problem of pain is considered as a complex biopsychosocial phenomenon, according to the results of a theoretical review, the main provisions of the concept of the multifactorial nature of pain are presented, which must be taken into account when selecting tools for assessing pain when working with gene dermatoses (epidermolysis bullosa) in childhood. Based on the analysis of Russian and foreign studies, a number of the most problematic areas in the assessment of pain in children are identified. Results and their discussion. According to the results of the analysis of a number of studies, the description of the main manifestations of epidermolysis bullosa and possible causes of pain is given. The biopsychosocial multifactorial nature of pain is considered, its neurobiological, psychological and functional consequences are presented, and on the basis of the research presented, conclusions are drawn for the first time about the principles of selecting the most effective tools for assessing pain and its impact on the vital activity of children with a chronic incurable disease. Pain assessment should include children’s previous experiences and expectations, coping strategies and pain relief resources, and communication features with caring adults. All forms of epidermolysis bullosa are characterized by pain and discomfort, and their elimination plays a key role in terms of the patient’s well-being and quality of life.

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Section: Introductionunclassified

The problem of pain and its assessment in children with epidermolysis bullosa

Troitskaya,

Golyaeva,

Orlova

2024

APPS

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Clinical and immunological characteristics of food allergy in different forms of inherited epidermolysis bullosa

Galimova,

Makarova,

Murashkin

2023

Russian Journal of Allergy

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BACKGROUND: Inherited epidermolysis bullosa is a severe orphan hereditary disease with a predominant lesion of the skin and mucous membranes. The study of the comorbid background, including food allergies, remains an urgent issue, given the difficulties that often arise in the treatment and formation of the diet in this category of patients. AIMS: to assess the frequency and nature of food allergies in children with inherited epidermolysis bullosa. MATERIALS AND METHODS: An open single-center randomized observational retrospective and prospective study included 165 patients aged 2 months to 17 years with an inherited epidermolysis bullosa. All patients were evaluated for an allergic history, determination of the levels of total IgE and specific serum IgE to the most significant food allergens (UniCAP System, Thermo Fisher Scientific), if necessary, a diagnostic elimination diet and diagnostic product administration were prescribed, based on the data obtained, the diagnosis of food allergy was confirmed or excluded. RESULTS: Among children suffering from inherited epidermolysis bullosa, confirmed food allergy was 13.9% of cases (in 13.4% in the group of children with dystrophic form of the disease, 15.2% in the group of children with a simple form of the disease). The main manifestations of food allergy in this cohort of patients were skin symptoms. Cow's milk proteins were the most frequent etiological factor of food allergy - 78.3%. Most children with food allergies had a high level of total IgE (87.5%). In children with non-IgE mediated form, high levels of total IgE were detected in 25% of cases, while these children were characterized by a severe course of the underlying disease or the presence of concomitant atopic dermatitis. Burdened heredity for allergic diseases turned out to be more typical for children with an IgE-mediated form of food allergy from the group of simple epidermolysis bullosa. CONCLUSIONS: Early detection of food allergies in children with inherited epidermolysis bullosa, as an aggravating factor in the course of the underlying disease, is necessary to optimize the tactics of dietary support for patients with inherited epidermolysis bullosa.

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Eye Involvement and Management in Inherited Epidermolysis Bullosa

Cited by 2 publications

References 64 publications

The problem of pain and its assessment in children with epidermolysis bullosa

The problem of pain and its assessment in children with epidermolysis bullosa

Clinical and immunological characteristics of food allergy in different forms of inherited epidermolysis bullosa

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